Download Congenital heart disease statistics

Congenital
heart
disease
statistics
2003
Sophie Petersen, Viv Peto and Mike Rayner
British Heart Foundation Health Promotion Research Group
Department of Public Health, University of Oxford
British Heart
Foundation
Statistics Database
www.heartstats.org
1
Scientific advisors for this supplement
Professor John Deanfield
BHF Vandervell Professor of Cardiology
Institute of Child Health,
University College, London
Professor Jane Somerville
Emeritrus Professor of Cardiology
Imperial College School of Medicine, London
Dr Barry Keeton
President, British Paediatric Cardiac Association
British Heart
Foundation
Statistics Database
www.heartstats.org
2
Contents
Page
Foreword
Summary
Introduction
1. Incidence and prevalence of
congenital heart disease
Table 1.1
Table 1.2
Table 1.3
Estimates of the numbers of babies born with
congenital heart disease by country, 2001, United Kingdom
Incidence of congenital heart defects, 1980-1997, Glasgow
Estimates of the number of people with congenital heart disease
(simple and complex), 2000 and 2010, United Kingdom
2. Mortality from congenital heart disease
Table 2.1
Table 2.2
Fig 2.2
Table 2.3
Deaths from congenital heart disease by sex, age and country,
2001, United Kingdom
Deaths from congenital heart disease by age-group, 1986-2001,
England and Wales
Percentage on deaths from congenital heart disease by age-group,
1986-2001, England and Wales
Number of cases, observed survival in infancy and
predicted survival throughout childhood by diagnostic category of
congenital heart disease, 1985-1999, Northern England
3. Morbidity from congenital heart disease
Table 3.1
Fig 3.1
SF-36 scores in adults with congenital heart disease
(with and without cyanosis) compared to the general population,
2000, West Midlands
SF-36 scores in adults with congenital heart disease
(with and without cyanosis) compared to the general population,
2000, West Midlands
4. Treatment of congenital heart disease
Table 4.1
Table 4.2
Table 4.3
Table 4.4
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Statistics Database
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3
5
6
7
10
12
13
14
15
16
17
17
18
19
21
21
22
Procedures for congenital heart disease,
April 2000 – January 2001, United Kingdom
25
Operations for congenital heart disease, 1977-1999, United Kingdom 25
Hospitalisations for congenital heart disease by age,
National Health Service hospitals, 2001/02, England
Paediatric and congenital cardiac services review: Standards table
26
27
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Foundation
Statistics Database
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4
Foreword
The formation of the human heart takes place during weeks six to twelve of fetal development.
The process is an intricate one which can be influenced by both genetic and environmental factors.
Until recently it was believed that eight children in every one thousand were born with a
developmental defect of their heart known as congenital heart disease. Congenital heart disease
statistics suggests that the earlier figure may represent a slight overestimate of the frequency of
congenital heart disease. The publication attempts to show the consequences of the various forms
of congenital heart disease, maps progress over the past forty years and draws attention to some
of the problems facing teenagers and adults whose heart defects have been treated surgically.
Traditionally, doctors have subdivided congenital heart defects into either simple or complex:
alternatively, into non-cyanotic or cyanotic conditions which reflect whether the baby is pink or
blue. Both classifications have merit in that children born with simple defects represent the large
majority of those affected. They tend to be non-cyanotic and have a good life expectancy. Some
simple congenital defects will correct themselves spontaneously whilst others can be treated nonsurgically. By contrast, those born with complex congenital heart defects are often cyanosed
(blue) and have a poor prognosis unless treated surgically. Although fewer in number, progress
over the past forty years has been spectacular. As a result there are now more adults than children
living with congenital heart disease
On average, one in five of those born with complex congenital heart defects used to survive into
adulthood. But, improvements in diagnosis, the development of heart lung bypass machines,
surgical innovations, better anaesthesia, better post operative care, as well as interventional
catheterisation, ensure that over 80% will now survive to become adults. Nevertheless, affected
individuals will need to be followed up by experts for the remainder of their lives. Consequently,
we have seen the development of a specialty known as grown-up congenital heart disease (GUCH).
Although the statistics contained in this publication give cause for celebration, there are many
challenges which lie ahead, which is why the British Heart Foundation is continuing to fund
research into the causes, diagnosis, prevention and treatment of congenital heart disease as well
as funding specialist cardiac liaison nurses, and provide information for those affected by congenital
heart disease either directly, as patients, or as family members.
Professor Sir Charles George
Medical Director
British Heart Foundation
British Heart
Foundation
Statistics Database
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5
Summary
■ In the UK there are about 4,600 babies born with congenital heart disease each year – one in
every 145 births.
■ The prognosis for babies born with congenital heart disease has improved dramatically over
the past 30 years.
■ At least three-quarters of babies with congenital heart disease are predicted to survive to
adulthood.
■ The prognosis for babies born with congenital heart disease varies widely depending on the
complexity of the congenital defect. Survival rates in a number of simple conditions are close
to 100%.
■ Treatment for congenital heart disease has evolved substantially over the last four decades,
with the development of many new surgical and cardiac catheterisation techniques.
■ Around 3100 operations and 725 interventional cardiac catheterisations are performed each
year on babies and children with congenital heart disease.
■ Mortality from operations performed on children with congenital heart disease has more
than halved since the late 1970’s.
■ There are a number of medical complications associated with congenital heart disease. Most
people with congenital heart disease have a life-long risk of infective endocarditis even after
repair. Those with more severe malformations, particularly those involving cyanosis (a chronic
shortage of oxygen in the blood), are at highest risk of a range of complications, including
kidney dysfunction and blood deficiencies.
■ Currently in the UK there are approximately 150,000 people aged 16 and over living with
congenital heart disease. Of these around 11,500 have the more complex forms of the disease,
which require life-long care.
■ It is estimated that the number of adults with congenital heart disease in the UK will grow by
25%, and the number with complex conditions by at least 50%, between 2000 and 2010.
■ As a result, over the next 10 years there will be a significant increase in the demand for
grown-up congenital heart disease (GUCH) services in the UK.
■ In the future, over 1,600 extra adults each year will require specialist GUCH services. It is
estimated that around 25% more consultant congenital cardiologists and surgeons are needed.
■ Quality of life in adults with simple congenital heart disease is similar to that found in adults
in the general population in all areas of life except physical functioning and general health
perception where it is lower.
■ However, the presence of cyanosis in adults with congenital heart disease is associated with a
more severe and more generalised reduction in quality of life.
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Foundation
Statistics Database
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6
Introduction
The aims of this publication
Heart disease is one of the most common forms of congenital abnormality in the UK. It affects
thousands of children and their families and can have serious, life-long consequences.
This supplement presents statistics on the burden of congenital heart disease in the UK, including
new estimates of the numbers of babies born each year with congenital heart disease. It aims to
characterise the burden of congenital heart disease both to individuals and to UK society as a
whole.
The publication contains four sections: on incidence and prevalence, mortality, morbidity and
treatment. Each section gives as far as possible data by age, sex and geographical region. Data by
socio-economic group and ethnic origin were not available and are therefore not included.
We originally aimed to include a section on the economic costs of congenital heart disease. However,
as no UK data on economic costs were available, this section was omitted.
What is congenital heart disease?
Congenital heart disease is a heart condition resulting from an abnormality in heart structure or
function that is present at birth. Most congenital heart conditions occur because the heart or its
valves and vessels are not properly formed. Additional defects, such as holes between the chambers
of the heart, may be present.
Types of congenital heart disease
There are many different types of congenital heart disease. These range from simple defects with
potentially minor consequences (for example, small ventricular septal defect), to complex
abnormalities with serious outcomes (for example, hypoplastic left heart syndrome). The type
and complexity of congenital heart disease depends on precisely how the structure and function
of the heart is affected in an individual.
Descriptions of the main types of congenital heart disease, together with diagrams to illustrate
each type of defect, can be found on the British Heart Foundation website at www.bhf.org.uk.1
Diagnosing congenital heart disease
Around 60% of congenital heart disease is diagnosed in babies (aged under a year), 30% in
children (aged 1-15), and 10% in adulthood (16 years and over).
As a result of routine screening during pregnancy, an increasing number of defects, particularly
the complex forms, are being diagnosed before birth.
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Statistics Database
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7
Symptoms of congenital heart disease in babies and children include failure to thrive, feeding
difficulties, slow growth, blueness, and dizziness or fainting. Signs include cyanosis, heart murmurs,
poor pulses and heart failure.
When congenital heart disease is suspected, a number of techniques are used to confirm the
diagnosis. Initial screening includes chest x-ray and electrocardiogram (ECG), with most
abnormalities then characterised reliably using echocardiography. However, in some cases, more
complex non-invasive and invasive tests such as Magnetic Resonance Imaging (MRI) and cardiac
catheterisation are also required.
What causes congenital heart disease?
In most cases of congenital heart disease the cause is unknown.
The main structure of the fetal heart is formed between the sixth and twelfth weeks of pregnancy,
and if normal development is interrupted at this time, defects result. Abnormalities in heart
valves, the myocardium or ductus arteriosis can also occur later on in pregnancy.
Some congenital heart defects relate to disease in the mother, for example if she has rubella
(German measles), a viral infection, diabetes or systemic lupus erythematosus (SLE – a disease
which affects the immune system) during pregnancy.
Drugs taken in pregnancy, such as some of those taken for epilepsy, lithium for depression, illegal
drugs or alcohol, are known to cause certain heart defects.
Some congenital heart defects are genetic. The incidence of congenital heart disease is increased
in the children of parents with congenital heart disease, and is higher if the mother rather than
the father is affected. There are also high levels of congenital heart disease found in babies with
a range of chromosomal syndromes, including Down’s syndrome.
The treatment of congenital heart disease
Some mild congenital heart conditions repair themselves without needing treatment. The majority
of cases do need treatment, and this varies depending on the type and complexity of the congenital
abnormality. Most conditions are treated with one or a combination of surgery, cardiac
catheterisation, or medication.
"Grown-up congenital heart disease"
In the 1950’s, less than one fifth of children born with major congenital heart disease reached
adulthood. The development of cardiac surgery for congenital cardiac malformations, and more
recently interventional catheterisation, means that over the past half century this poor prognosis
has been reversed. Now around 80% of those born with congenital heart disease can expect to
reach adulthood. Grown-up congenital heart disease (GUCH) is the term used in the UK to
describe the range of heart conditions experienced by people aged over 16 years who were born
with congenital heart disease.
Methods for this publication
In compiling this publication we have aimed to investigate all possible sources of recent data
relating to the burden of congenital heart disease in the UK. Compared to the data available for
earlier publications in this series (such as Coronary heart disease statistics: Heart failure supplement
2002), the amount of data on this topic is limited. There are no national studies available; only
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Foundation
Statistics Database
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8
few sources supply data by age, sex or geographical location; and no data are published to show
ethnic or socio-economic variation.
While various sources of information have been used in compiling this supplement, including
routine national statistics, we have drawn heavily on a small number of local databases, such as
the Northern Region Paediatric Cardiology Database. We have calculated national estimates
from these studies, which gave the widest coverage in terms of sex, age, geographical location,
and used the most valid and reliable methods of collection. We have not included data from
outside the UK but have aimed to include the most recent data available.
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Foundation
Statistics Database
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1.
For more detailed descriptions of congenital heart disease conditions, see specialist texts, for example: Anderson RH, Macartney FJ,
Shinebourne EA and Tynan MJ (1987) Paediatric Cardiology. Churchill Livingston:Edinburgh, or Archer N and Burch M (1998)
Paediatric Cardiology: An introduction. Chapman & Hall Medical: London.
1. Incidence and
prevalence of
congenital
heart disease
Incidence
The incidence of congenital heart disease is the rate of new cases of congenital heart disease,
usually expressed as the number of babies born with congenital heart disease per 1,000 live
births.
Data from the Northern Region Paediatric Cardiology Database show that between 1985 and
1999 there were 5.2 cases of congenital heart disease diagnosed in infancy for every 1,000 live
births.
However, not all cases of congenital heart disease are diagnosed in infancy. Incidence rates based
on diagnoses in the first 12 months of life will, therefore, be an underestimate of the true incidence.
Further data from the Northern Region Paediatric Cardiology Database suggest around 1 in 4
cases of congenital heart disease in the UK are diagnosed later in childhood1. Based on these
figures, we estimate the incidence rate for congenital heart disease in the UK to be 6.9/1,000, or
one in every 145 babies born2.
From this incidence rate we estimate that there are just over 4,600 babies born with congenital
heart disease in the UK each year (Table 1.1).
The Glasgow Register of Congenital Anomalies is a population-based registry covering all births
within the Greater Glasgow Health Board in Scotland. All cases of congenital anomaly identified
in live births, stillbirths and induced abortions following prenatal diagnosis are recorded in the
register, along with a classification of the main type of anomaly. Between 1980 and 1997, the
overall incidence of congenital heart defects in the Glasgow Register fell by around 25% from
8.3/1,000 to 6.2/1,000 live and still births3 (Table 1.2).
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Statistics Database
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10
Prevalence of congenital heart disease in adults
The precise prevalence of congenital heart malformations in the adult population is unknown.
However, estimates can be made of the current population of adults with congenital heart disease
in the UK, combining the known birth rate and published incidence and survival rates.
The British Cardiac Society Working Party on grown-up congenital heart disease (GUCH) recently
conducted such an analysis. It was estimated that in 2000 there were just under 150,000 adults
with congenital heart disease in the UK. Of these, around 11,500 had the more complex forms of
the disease, requiring life-long expert supervision and intervention4 (Table 1.3).
The Working Party further estimated that by the year 2010 there would be over 185,000 adults
in the UK living with congenital heart disease (over 17,000 with the complex forms), a rise of
around 25% in simple and 50% in complex conditions since 2000 (Table 1.3).
An analysis based on data from the Northern Region Paediatric Database predicted an even
greater growth in the numbers of adults with congenital heart disease over the next decade. This
estimates there will be an extra 1,600 adults per year in the UK, living with moderate and complex
forms of congenital heart disease and needing long term follow up care5.
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Statistics Database
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11
1.
Between 1985 and 1999 in the Northern Region of the National Health Service, 1,590 cases of congenital heart disease were
diagnosed in infancy (under 1 year) and 605 cases diagnosed in children (aged 1-15 years). Correcting for under ascertainment in
those aged 5-15 years, added another 73 post infant diagnoses. The most common abnormalities diagnosed after infancy were
ventricular septal defect, patent ductus arteriosus, atrial septal defect and pulmonary valve stenosis. See Wren C and O’Sullivan J
(2001) Survival with congenital heart disease and need for follow up in adult life. Heart;85:438-43.
2.
Data from the USA suggest that at least 10% of cases seen in a GUCH clinic are not diagnosed until adulthood - in particular
secundum atrial septal defect, Ebstein’s anomaly and congenitally corrected transposition. (Webb G and Williams R (2001) 32nd
Bethesda Conference: Care of the Adult with Congenital Heart Disease. Journal of the American College of Cardiology; 37: 116198). Assuming this is also the case in the UK, the true incidence rate of congenital heart disease is a further 10% higher, around
7.6/1,000 (or 1 in 132 births, around 5,000 babies born in the UK per year).
3.
The Glasgow Registry excludes heart defects not requiring surgery (for example, mild ventricular septal defects) and defects occurring
in babies born at less than 37 weeks, or weighing less than 2,500g. This means that incidence rates for congenital heart disease
reported by the Glasgow Register are lower than those reported by the Northern Region Paediatric Cardiology Database, which is
likely to be a truer reflection of the underlying incidence.
4.
For a list of conditions defined as complex in this analysis, see source: Report of the British Cardiac Society Working Party (2002)
Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart
disease in the UK. Heart;88 (Suppl I): i1-i14.
5.
Wren C and O’Sullivan J (2001) Survival with congenital heart disease and need for follow up in adult life. Heart;85:438-43.
3,092
273
114
3,479
52,527
21,962
669,123
Diagnosed in infancy
(5.2/1,000 births)
594,634
Total births
Sources:
General Register Office (2002) Annual Report 2001. Statistics and Research Agency: Northern Ireland.
General Register Office (2002) Edinburgh, Scotland http://www.gro-scotland.gov.uk;
Office for National Statistics (2002) Birth statistics (Series FM1 no.30). Office for National Statistics:London;
Wren C and O'Sullivan J (2001) Survival with congenital heart disease and need for follow-up in adult life. Heart;85:438-43:
1,138
38
89
1,011
Diagnosed 1-15 years
(1.7/1,000 births)
4,617
152
362
4,103
Total
(6.9/1,000 births)
Number of babies born with congenital heart disease
Estimates of the numbers of babies born with congenital heart disease by
country, 2001, United Kingdom
Incidence rates, in parentheses, are from the Northern Region Paediatric Cardiology Database, 1985-1999.
UK
Northern Ireland
Scotland
England and Wales
Table 1.1
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Foundation
Statistics Database
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12
British Heart
Foundation
Statistics Database
www.heartstats.org
13
Table 1.2
Incidence of congenital heart defects,
1980-1997, Glasgow
Year of birth
Number live and still births
with congenital heart defects
Rate/1,000
live and still births
1980
1981
1982
1983
1984
1985
1986
1987
1988
1989
1990
1991
1992
1993
1994
1995
1996
1997
112
121
137
90
98
97
71
76
82
73
68
79
62
62
71
67
69
68
8.3
9.0
10.6
7.1
7.7
7.4
5.5
5.9
6.4
5.9
5.5
6.2
5.0
5.2
6.2
6.0
6.2
6.2
Total
1,503
6.7
Source:
The Greater Glasgow NHS Board Congenital Anomalies Register, personal communication.
16,620,000
17,260,000
7,550,000
1960-1980
1980-1990
7,550,000
1980-1990
1940-1960
17,260,000
1960-1980
33,980
77,680
74,790
11,325
25,890
24,930
Number born with
congenital heart disease
90%
90%
90%
70%
50%
20%
First year
survival rate
30,582
167,805 in year 2010
69,912
137,223 in year 2000
67,311
5,663
17,218 in year 2010
9,062
11,555 in year 2000
2,493
Survivors at
18 years
148,778 in the year 2000
185,023 in the year 2010
90%
90%
90%
50%
35%
10%
18 year
survival rate
All congenital heart disease
30,582
69,912
67,311
7,928
12,945
4,986
Survivors at
12 months
Source:
Report of the British Cardiac Society Working Party (2002) Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK.
Heart;88 (Supp I):i1-i14.
These calculations are by AG Stuart and are based on the methods used to calculate the same population for the USA, at the 32nd Bethesda Conference "Care of the adult survivor with congenital heart disease, October 2000.
The incidence of complex congenital heart disease is assumed to be 1.5 per 1,000 live births, and simple congenital heart disease 4.5 per 1,000 live births. For details of conditions considered to be complex with speical risks
and complications see source.
Simple congenital heart disease
(Incidence - 4.5/1,000 births)
16,620,000
Number of births
in the UK
1940-1960
Date of birth
Estimates of the number of people with congenital heart disease (simple and complex),
2000 and 2010, United Kingdom
Complex congenital heart disease
(Incidence - 1.5/1,000 births)
Table 1.3
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Foundation
Statistics Database
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14
2. Mortality from
congenital
heart disease
National mortality statistics
In 2001, 811 deaths due to congenital heart disease were recorded in the UK (Table 2.1).
Just over a third of these deaths were in babies aged less than a year, one in seven were in children
and adolescents (aged 1-19 years) and the remaining half were in adults (aged 20 years and over).
Data from England and Wales show that the proportion of deaths from congenital heart disease
occurring in babies has declined markedly over the last two decades. In 1986 death was most
common in infancy, with just under 60% of deaths from congenital heart disease occurring in
babies aged less than a year. By the early 1990’s death from congenital heart disease was most
common in adults aged 20 years and over (Table 2.2 and Fig 2.2).
Survival
Data from the Northern Regional Paediatric Cardiology Database show that 82% of babies
diagnosed with congenital heart disease survive the first year of life (Table 2.3).
However, first year (infant) survival rates vary widely depending on the nature of the congenital
heart defect. In the Northern Regional Paediatric Cardiology Database the lowest first year survival
rates were observed in babies with hypoplastic left heart, truncus arteriosus and more severe forms
of pulmonary artresia, and the highest in babies with mild cases of aortic or pulmonary stenosis,
ventricular septal defect, and congenitally corrected transposition of the great arteries (Table 2.3).
It should be noted that these data are based on births going as far back as 1985. With major
advances to treatment over the past decade, babies born today with the more serious heart defects
are likely to have much higher one-year survival rates than these data suggest. For example,
today around 50% of babies born with hypoplastic left heart survive infancy1.
Combining data on observed infant survival with estimates of the likely childhood survival rates
(from 1 year to 16 years), it is predicted that overall 78% of babies diagnosed with congenital
heart disease will survive to adulthood2 (Table 2.3).
British Heart
Foundation
Statistics Database
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15
1.
Andrews R, Tulloh R, Sharland G, Simpson J, Rollings S, Baker E, Qureshi S, Rosenthal E, Austin C and Anderson D (2001)
Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis. Archives of Disease in
Childhood;85:474-77.
2.
This prediction is based on published survival rates in patients who mostly had operations 15 or more years ago when they were in
infancy. Marked improvements in care in the last 15 years mean that this prediction is likely to be an underestimate of the true
survival rate into adulthood. Data from the USA suggest this is likely to be around 85%. See Perloff JK and Warnes CA (2001)
Challenges posed by adults with repaired congenital heart disease. Circulation;103:2637-43.
Table 2.1
Deaths from congenital heart disease by sex, age
and country, 2001, United Kingdom
All ages
Under 1
1-9
10-19
20-44
45-69
70 and over
England and Wales
Males
Females
Total
371
351
722
107
133
240
30
22
52
31
14
45
84
52
136
78
66
144
41
64
105
Scotland
Males
Females
Total
34
31
65
16
13
29
2
3
5
4
3
7
8
5
13
3
4
7
1
3
4
Northern Ireland
Males
Females
Total
12
12
24
2
6
8
1
1
2
1
1
2
4
1
5
2
2
4
2
1
3
UK
Males
Females
Total
417
394
811
125
152
277
33
26
59
36
18
54
96
58
154
83
72
155
44
68
112
ICD10 codes Q20-28
Figures for deaths under 1 year include data on neonatal deaths (deaths under 28 days) which are published separately in England and
Wales (DH3 series).
Sources:
Office for National Statistics (2002) Mortality statistics: cause (Series DH2 no.28). HMSO:London;
Office for National Statistics (2003) Mortality statistics: childhood, infant and perinatal (Series DH3 no. 34). HMSO:London;
General Register Office: Edinburgh, Scotland http://www.gro-scotland.gov.uk/grosweb/grosweb.nsf/pages/refsct6;
General Register Office (2002) Annual Report 2001. Statistics and Research Agency: Northern Ireland.
British Heart
Foundation
Statistics Database
www.heartstats.org
16
Table 2.2
Deaths from congenital heart disease by agegroup, 1986-2001, England and Wales
1986
1987
1988
1989
1990
1991
1992
1993
1994
1995
1996
1997
1998
1999
2000
2001
Source:
All ages
Under 1
1-9
10-19
20 & over
1,015
960
960
1,401
1,304
1,291
1,235
1,011
939
907
868
901
837
805
787
722
584
565
582
693
613
610
601
397
370
350
362
337
312
314
293
240
103
81
86
207
181
173
132
115
103
79
80
85
65
55
50
52
34
43
42
57
62
59
64
55
45
49
47
66
46
52
43
45
294
271
250
444
448
449
438
444
421
429
379
413
414
384
401
385
Office for National Statistics (2002) Mortality statistics: cause (Series DH2 no.28). HMSO:London and earlier editions;
Office for National Statistics (2003) Mortality statistics: childhood, infant and perinatal (Series DH3 no. 34). HMSO:London
and earlier editions.
Figure 2.2
Percentage of deaths from congenital heart
disease by age-group, 1986-2001, England and
Wales
% deaths from congenital heart disease
70
60
20 years
and over
50
40
Under 1 year
30
20
10
1-9 years
10-19 years
0
1986
British Heart
Foundation
Statistics Database
www.heartstats.org
17
1987
1988
1989
1990
1991
1992
1993
1994
1995
1996
1997
1998
1999
2000
2001
Table 2.3
Number of cases, observed survival in infancy
and predicted survival throughout childhood by
diagnostic category of congenital heart disease,
1985-1999, Northern England
Diagnostic category
Hypoplastic left heart
Truncus arteriosus
Pulmonary atresia with intact ventricular septum
Aortic stenosis with intervention or death in infancy
Tricuspid atresia
Complete atrioventricular septal defect
Mitral regurgitation
Double inlet ventricle
Ventricular septal defect with intervention or death in infancy
Pulmonary atresia with ventricular septal defect
Total anomalous pulmonary venous connection
Transposition of the great arteries
Partial atrioventricular septal defect
Mitral atresia
Pulmonary stenosis with intervention or death in infancy
Miscellaneous
Atrial septal defect
Coarctation of aorta
Tetralogy of Fallot
Patent ductus arteriosus
Congenitally corrected transposition of the great arteries
Ventricular septal defect with no intervention
Aortic stenosis with no intervention
Pulmonary stenosis with no intervention
Total
Number
of cases*
54
35
21
36
23
104
7
30
182
39
33
113
29
10
46
69
84
90
113
95
17
577
38
97
Observed survival
to 1 year
%
0
34
43
47
56
56
57
60
66
72
73
77
79
80
83
83
87
88
89
94
100
100
100
100
Predicted survival
to 16 years
%
0
31
31
37
31
54
54
43
64
48
71
67
72
32
81
79
84
86
84
93
96
100
94
97
1942
82
78
*Number of live born diagnosed with congenital heart disease by the age of 12 months.
Source:
Wren C and O'Sullivan J (2001) Survival with congenital heart disease and need for follow up in adult life. Heart; 85:438-43.
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3. Morbidity
from
congenital
heart disease
Medical complications associated with congenital
heart disease
There are a number of medical complications associated with congenital heart disease. These
include skeletal deformities, joint and bone problems, renal (kidney) dysfunction, blood cell
disorders and bleeding tendencies, gallstones and acne.
The risk of developing these complications depends on the type and severity of congenital heart
abnormality present at birth. Where cyanosis (a chronic shortage of oxygen in the blood) is
present, these complications are much more likely to develop1.
Most babies, children and adults with congenital heart disease have a life-long risk of infective
endocarditis: an infection usually affecting a heart valve or a blood vessel. While infective
endocarditis is rare in the general population, people with a heart defect are at a greater risk of
developing it, and need to take preventive measures such as taking antibiotics prior to dental
treatment, childbirth, body piercing and surgery. Infective endocarditis accounts for around 4%
of admissions to a specialised unit for grown-up congenital heart disease patients in the UK2.
Quality of life
The Short-form 36 (SF-36) questionnaire measures health related quality of life in eight areas of
life, including physical, mental and social functioning. Patients registered at the Adult Congenital
Heart Disease clinic in Birmingham were surveyed using the SF-36 to measure quality of life in
adults with congenital heart disease.
Compared to adults of a similar age in the general population, adults with simple congenital
heart disease had significantly lower SF-36 scores for only two dimensions: physical functioning
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and overall general health perception.
Patients with cyanosis had lower SF-36 scores than patients without cyanosis. The presence of
cyanosis was associated with a more severe and more generalised reduction in quality of life.
Patients with cyanosis reported significantly lower levels of physical, social and mental functioning
compared to the general population and to patients without cyanosis (Table and Fig 3.1)3.
1.
For details of the impact of cyanosis and its associated morbidities, see Recommendations of the Task Force of the European Society
of Cardiology (2003) Management of Grown-ups with Congenital Heart Disease. European Heart Journal;24:1035-84.
2.
Li W and Somerville J (1998) Infective endocarditis in the grown-up congenital heart (GUCH) population. European Heart
Journal;19:166-73.
3.
Results from a similar study on health related quality of life in the Netherlands, confirm the impact of congenital heart disease on
physical functioning in adulthood. Here adults with previously operated complex congenital heart disease experienced limitations
only in the physical dimensions of quality of life compared to the general population. Kamphuis M, Ottenkamp J, Vliegen HW,
Vogels T, Zwinderman KH, Kampuis R, Verloove-Vanhorick (2002) Health related quality of life and health status in adult survivors
with previously operated complex congenital heart disease. Heart; 87:356-362.
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Table 3.1
SF–36 scores in adults with congenital heart
disease (with and without cyanosis) compared
to the general population, 2000, West Midlands
Adults with congenital heart disease
Cyanotic
Acyanotic
Quality of life category
Physical functioning
Role-physical
Role- emotional
Social functioning
Mental health
Energy/vitality
Pain
General health perception
Bases
General population
48.1
53.6
64.1
65.3
60.6
46.7
68.5
39.0
84.6
83.8
78.8
82.8
73.5
62.5
86.9
66.2
92.6
88.5
82.8
88.4
72.9
60.3
83.8
76.3
18
54
1,134
Lower scores on the SF-36 represent poorer quality of life. Scores can range from 0-100.
General population scores are SF-36 UK normative data for people aged 30-34 years (from the Oxford Healthy
Lifestyle Survey,1991/92, HSRU, Oxford). The median age of adults with congenital heart disease in this study is 31 years.
Source:
Lane D, Lip G and Millane T (2002) Quality of life in adults with congenital heart disease. Heart; 88:71-75.
Fig 3.1
100
SF–36 scores in adults with congenital heart
disease (with and without cyanosis) compared
to the general population, 2000, West Midlands
Adults with congenital heart disease - cyanotic
Adults with congenital heart disease - acyanotic
General population
90
80
SF-36 score
70
60
50
40
30
20
10
0
Physical
functioning
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Role-physical Role- emotional
Social
functioning
Mental health Energy/vitality
Pain
General health
perception
4. Treatment of
congenital
heart disease
Not all congenital heart disease requires treatment. Some mild congenital heart defects repair
themselves. For example, in cases of small ventricular septal defect, the “hole in the heart” often
becomes smaller, eventually closing by itself as the child grows. However, the majority of congenital
heart conditions do need treatment, and this varies depending on the type and complexity of the
congenital defect.
The treatment of congenital heart disease has developed dramatically over the past 40 years.
This can be illustrated using a condition called transposition of the great arteries as an example.
In transposition of the great arteries, the two main arteries of the heart, the aorta and the pulmonary
artery, are transposed, or switched, so that de-oxygenated rather than oxygenated blood is pumped
around the body. Without treatment 90% of babies born with this condition die within 12
months.
In the early 1960’s treatment for transposition of the great arteries was revolutionised by the first
ever interventional catheterisation technique, the balloon septostomy. This was superceded later
that decade by an operation known as the Mustard or Senning. This had excellent results (less
than 5% mortality in childhood) and continued to be performed until the 1980’s. However, it
became clear after long-term follow up, that while the Mustard/Senning operation gave babies
with transposed great arteries a much better prognosis during childhood, morbidity and mortality
in early adulthood were high. A new operation, the arterial switch, which gave an "anatomical
repair", was introduced in the early 1990’s. This had much lower success rates to begin with, but
was developed in the expectation of better late results compared to the Mustard/Senning treatment.
This proved to be the case, together with marked improvements in surgical survival. The arterial
switch is now the standard treatment for babies born with transposition of the great arteries.
This rapid evolution in the treatment of congenital heart disease, together with considerable
variation in practice at the UK’s specialized centres for paediatric and GUCH patients, means the
availability of simple nationally representative data on the treatment of congenital heart disease
is limited.
Procedures for congenital heart disease
Data published by the Central Cardiac Audit Database show that between April 2000 and January
2001, just over 2,300 operations were performed in the UK’s 14 specialist paediatric congenital
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heart disease units. Overall the mortality rate from these operations was 5.1%. During the same
nine-month period around 550 interventional cardiac catheterisation procedures were also carried
out (Table 4.1).
From these figures we estimate around 3,100 operations and 725 interventional cardiac
catheterisations are performed each year on babies and children with congenital heart disease1.
Trend data suggest that the number of operations performed on children with congenital heart
disease increased by around 15% between the late 1970’s and the late 1990’s2. Over the same
period mortality from these operations more than halved (Table 4.2).
No national data on procedures for grown-up congenital heart disease (GUCH) are available.
However, unpublished data from a specialist GUCH unit in London which cares for over 2,400
patients, show that between 1999 and 2002, 342 procedures were carried out, of which just
under one-third were transcatheter rather than surgical procedures3.
Further data from the patient register at the Adult Congenital Heart Disease clinic in Birmingham
suggest around three-quarters of adults with complex congenital heart disease have had surgery
to treat their congenital heart defect. Around one quarter are treated with drugs, with no clinical
need for surgery. Of those who have been treated by surgery, further surgery is likely to be
needed in two-thirds of cases, with only one-third of cases categorised as surgically “cured”. A
small minority, less than one in twenty, have more serious conditions, which require organ
transplantation4.
Hospital admissions
Hospital episode statistics show that in 2001/02 there were 15,700 hospitalisations in National
Health Service hospitals in England where congenital heart disease was the principal diagnosis.
Overall there were over 72,000 days of inpatient care due to congenital heart disease. These
represent around 0.1% of all inpatients cases and all inpatients days in England (Table 4.3).
In around 70% of the hospitalisations for congenital heart disease the patient was a child aged 014 years. In 5% of hospitalisations, the patient was 60 years or above. The mean age of inpatients
was 16 years.
Review of paediatric and congenital cardiac services
In response to public concerns and inquiries into the quality of care at a number of specialist
centres in the UK, most notably the Bristol Royal Infirmary, the Department of Health initiated
a review of NHS paediatric and congenital cardiac services. The initial report of this review was
published in November 20025. The report set out twelve standards for services for the care of
patients with congenital heart disease. As well as standards relating to the quality of medical and
surgical care, the need for screening and early diagnosis, and the importance of information and
support for patients and parents, the review included a standard to recognise the need for ageappropriate care and services for adults with congenital heart disease (Table 4.4).
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Progress towards these standards will be monitored in future publications of the British Heart
Foundation Statistics group6.
Future demand for services
The Review of Paediatric and Congenital Cardiac Services predicts that the demand for services
for children born with congenital heart disease will increase slightly over the next 10-15 years.
Forecasts of a slight increase in the birth rate will mean an increase in the number of babies born
with congenital heart disease of around 5% by 20185,7.
In contrast to the relatively stable demand for paediatric services, a number of studies indicate
there will be a significant increase in the demand for grown-up congenital heart disease services
in future years. These increases reflect the substantial and continuing improvements in childhood
survival rates over the last two decades.
The Review of Paediatric and Congenital Cardiac Services follows the approach of the British
Cardiac Society Working Party on grown-up congenital heart disease8 which predicted there will
be a 50% increase in the number of adults with complex congenital heart disease between 2000
and 2010 (an increase of around 5,600 adults). As a proportion of these adults will require
further surgery, and all will require long-term medical care, the Review estimates an increase in
consultant congenital cardiologist and surgeon capacity of around 25% (approximately 20
cardiologists and 2-3 surgeons) is required5.
Data from an alternative analysis by cardiologists based at the Freeman Hospital in Newcastle,
suggest the Department of Health figures may underestimate the potential growth in demand for
GUCH services in the UK. Based on observed cases, predicted survivors and the need for followup in a broad range of specific congenital heart disease conditions, this study predicts that there
will be just over 1,600 extra adults each year (or 16,000 between 2000 and 2010) requiring the
follow-up care of a specialised GUCH clinic.
1.
These figures do not include operations for grown-up congenital heart disease, or diagnostic cardiac catheterisations at any age.
2.
These data are from the congenital section of the UK Cardiac Surgical Register, collected by the Society of Cardiothoracic Surgeons
of Great Britain and Ireland. This section of the register was abandoned in 2000 due to growing concerns over data quality and the
inability of the register to reflect practice as new techniques evolved . The congenital section of the UK Cardiac Surgical Register was
replaced by the Central Cardiac Audit Database Project (CCAD), which is a combined cardiological and cardiac surgical database
managed by the NHS Information Authority. The first CCAD report on procedures for congenital heart disease was published in
2001 and is summarised in Table 4.1. Because of differences in methodology, the data in Tables 4.1 and 4.2 are not strictly comparable.
3.
These data come from the Grown-up Congenital Heart Unit at the Heart Hospital, London. Personal communication.
4.
See Table 1 in Lane D, Lip G and Millane T (2002) Quality of life in adults with congenital heart disease. Heart 2002;88:71-75.
5.
Department of Health (2002) Report of the Paediatric and Congenital Cardiac Services Review Group. See: www.doh.gov.uk/
childcardiac/reviewnov02.htm
6.
The British Heart Foundations Statistics team are based at the University of Oxford and are responsible for the www.heartstats.org
website and all British Heart Foundation Statistics publications.
7.
Likely advances in fetal cardiology over the next 10-15 years will also mean more conditions are detectable before birth, giving
parents the option of termination, as well as creating the opportunity for optimal planning of neonatal interventions. Medical
progress as a whole means there will be more patients for whom a medical or surgical intervention will be appropriate. The Review
assumes these opposing tendencies are likely to cancel each other out, in terms of future demands for services.
8.
Report of the British Cardiac Society Working Party (2002) Grown-up congenital heart (GUCH) disease: current needs and provision
of service for adolescents and adults with congenital heart disease in the UK. Heart; 88 (Suppl I):i1-i14. See Table 1.4.
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Table 4.1
Procedures for congenital heart disease, April
2000 - January 2001, United Kingdom
Number of procedures
Total mortality
%
2,326
1,525
797
5.1
5.4
4.5
542
0.6
Cardiac surgery
Bypass
Non-bypass
Interventional catheter procedures
Figures for bypass and non-bypass operations do not sum to 2,326 due to missing data on type of operation for four operations. Data
collated and reported by the Central Cardiac Audit Database.
Source:
Society of Cardiothoracic Surgeons of Great Britain and Ireland and the British Paediatric Cardiac Association (2001)
First paediatric report. See www.scts.org/file/Paed20002001.pdf
Table 4.2
1977
1978
1979
1980
1981
1982
1983
1984
1985
1986
1987
1988
1989
1990
1991
1992
1993/4
1994/5
1995/6
1996/7
1997/98
1998/99
1999/00
Operations for congenital heart disease, 19771999, United Kingdom
Number of operations
Total mortality
%
3,344
3,385
3,275
3,472
3,501
3,392
3,719
3,398
3,554
3,698
3,696
3,809
3,474
3,632
3,788
3,983
3,468
3,218
4,286
3,890
3,873
3,836
3,876
10.1
11.1
10.5
11.0
8.9
9.9
10.4
8.7
7.6
8.4
8.4
8.2
8.9
6.7
7.8
7.3
6.1
6.1
5.7
4.1
3.9
4.7
4.2
Data are from the UK Cardiac Surgical Register, collected by the Society of Cardiothoracic Surgeaons of Great Britain and Ireland.
Source:
British Heart
Foundation
Statistics Database
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Society of Cardiothoracic Surgeaons of Great Britain and Ireland (2003) http://www.scts.org
5,651,066
1,655,961
Source:
Department of Health (2003) Hospital Episode Statistics. http://www.doh.gov.uk/hes/
ICD codes (10th revision) in parentheses.
Finished consultant episodes; ordinary and day cases combined.
All diagnoses
3,798
11,095
All congenital heart disease (Q20-28)
150
1,374
133
287
225
310
28
486
805
15-59
1,559
4,002
683
798
760
2,657
311
220
105
0-14
Episodes of care
2,510,211
618
1
286
5
24
35
40
8
110
109
60-74
2,483,471
183
1
54
1
14
11
22
1
67
12
75 & over
12,357,360
15,700
1,711
5,716
822
1,124
1,033
3,031
348
884
1,031
All
51,111,153
72,141
9,804
24,962
3,777
6,011
4,422
13,910
1,724
3,202
4,329
Total days
Days in hospital
Hospitalisations for congenital heart disease by age, National Health Service hospitals,
2001/02, England
Congenital malformations of cardiac chambers and connections (Q20)
Congenital malformations of cardiac septa (Q21)
Congenital malformations of pulmonary and tricuspid valves (Q22)
Congenital malformations of aortic and mitral valves (Q23)
Other congenital malformations of heart (Q24)
Congenital malformations of great arteries (Q25)
Congenital malformations of great veins (Q26)
Other congenital malformations of peripheral vascular system (Q27)
Other congenital malformations of circulatory system (Q28)
Primary Diagnosis
Table 4.3
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Table 4.4
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Paediatric and congenital cardiac services
review: Standards table
Review Area
Review Standard
The context of care
■ The clinical team. The congenital heart service should function
as a team. It should be well led with responsibilities shared across
it. As a result, patients should receive seamless, expert and
continuous care that takes account of their changing needs.
■ A quality service. Clinical practice should be evaluated in a
systematic way, ensuring that key lessons are identified and
disseminated, and that clear and supportive lines of accountability
are in place.
■ Child-centred environment. Children should be cared for in an
environment that recognises their special needs.
Information and
consent
■ Patients and parents should be supported in understanding
their condition and treatment options, and in giving informed
consent at every stage of treatment.
The patients journey
■ Screening, assessment and diagnosis. Screening and diagnosis
should identify congenital heart abnormalities, and ensure that
patients are referred to the relevant specialist, as early as possible.
Accurate diagnosis should follow promptly.
■ Medical and surgical care. Medical and surgical procedures should
be carried out in a way that is safe for patients and which
maximises quality of care.
Joined up care
■ Going home. Patients/parents should continue to receive the
support and quality of care that will enable them/their child to
achieve as complete a recovery as possible, after or between
interventions.
■ Outreach services and the clinical network. Specialist centres
should work with local paediatric or cardiology services so that
as much cardiac diagnosis and care as possible is provided in a
network of locally accessible outreach services. The patient and
family should be asked to travel to the specialist centre only when
essential.
Growing up
■ Age-appropriate care. Patients should receive appropriate care
at each stage in their personal development and any changes in
location or specialist, because, for example, of a move to an adult
service, should be smoothly managed with the patient’s/parent’s
full understanding and agreement.
■ Services for adults with congenital heart disease. Young people
and adults with congenital heart disease should receive
appropriate services depending on the complexity of their
condition. It is recognised that these patients fall into two
categories: patients with simple lesions and follow-ups; and
complex cases, which require care from a dedicated team of more
specialist medical services.
Support for parents
and families
■ Facilities for parents and families. There should be adequate
facilities and accommodation available for parents when a child
is admitted to hospital.
■ Bereavement support. Parents/next of kin should be supported
after the death of a patient and given information that will help
them understand why the death occurred.
Source:
Department of Health (2002) Report of the Paediatric and Congenital Cardiac Services Review Group www.doh.gov.uk/
childcardiac/reviewnov02.htm
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